Acute presentation of giant sporadic vestibular schwannoma with massive hemorrhage: A critical review with a case illustration.

Introduction: Hemorrhage in vestibular schwannoma (VS) is a rare but potentially devastating complication, owing to the proximity to the brainstem and small space in the posterior fossa. Research question: it is a challenge to manage such patients, coming in emergency in comatose state and whether early intervention can reverse the consequence or not. Material and methods: A 42-year-old male presented in emergency room (ER) with sudden onset of severe headache followed by loss of consciousness, with GCS- 3 (E1V1M1) and mid-dilated fixed pupil. Computer tomography (CT scan) head revealed a large hematoma in the right cerebellopontine angle (CP) with hydrocephalus, requiring urgent CSF diversion (External Ventricular Drain) in the ER. Subsequently microsurgical excision of the tumor was done after few days, once patient has shown improvement in GCS. Histopathology confirmed it as hemorrhagic VS. Post-operatively, he had right HB (House- Brackemann) grade IV facial weakness which could not be appreciated in preoperative phase. He gradually recovered well and was able to walk without support at the time of discharge. Result: At 4 months follow up, facial weakness slightly improved to HB grade III, and patient was doing his daily activity without difficulty. Follow-up magnetic resonance imaging (MRI) imaging showed a small residue near internal acoustic meatus (IAM). Discussion and conclusion: Hemorrhage in VS is associated with increased morbidity and mortality; and few times, urgent intervention can save life despite of comatose state of the patient.

Surgical Outcome of Large Solid Posterior Fossa Hemangioblastoma without Preoperative Embolization.

BACKGROUND: Large solid hemangioblastoma in the posterior fossa has an abundant blood supply as an arteriovenous malformation. The presence of adjacent vital neurovascular structures makes them vulnerable and difficult to operate. Complete surgical resection is always a challenge to the neurosurgeon. MATERIAL AND METHOD: We share the surgical difficulties and outcome in this case series of large solid hemangioblastomas without preoperative embolization as an adjunct. This study included five patients (three men and two women, with a mean age of 42.2 years). Preoperative embolization was attempted in one patient but was unsuccessful. All the patients have headache (100%) and ataxia (100%) as an initial symptom. A ventriculoperitoneal shunt was inserted in one case before definite surgery due to obstructive hydrocephalus. The surgical outcome was measured using the Karnofsky Performance Status (KPS) score. RESULT: The tumor was excised completely in all the cases. No intra- and postoperative morbidity occurred in four patients; one patient developed transient lower cranial nerve palsy. Mean blood loss was 235 mL, and no intraoperative blood transfusion was needed in any case. The mean follow-up period was 14.2 months. The mean KPS score at last follow-up was 80.One patient had a KPS score of 60. CONCLUSION: Our treatment strategy is of circumferential dissection followed by en bloc excision, which is the optimal treatment of large solid hemangioblastoma. The use of adjuncts as color duplex sonography and indocyanine green video angiography may help complete tumor excision with a lesser risk of complication. Preoperative embolization may not be needed to resect large solid posterior fossa hemangioblastoma, including those at the cerebellopontine angle location.

Tumor Occupancy Ratio-An Imaging Characteristic Prognosticating the Surgical Outcome of Benign Intradural Extramedullary Spinal Cord Tumors.

BACKGROUND: Intradural extramedullary (IDEM) spinal cord tumors are two thirds of all spinal tumors. We have prospectively analyzed the importance of the tumor occupancy ratio as a factor for predicting the course of the disease and in prognosticating the surgical outcome in patients with IDEM tumors. METHODS: We prospectively analyzed 44 consecutive cases of IDEM tumors, diagnosed as cervical, thoracic, and lumbar IDEM tumors (excluding conus/cauda equina lesion) by magnetic resonance imaging (MRI), that were operated on at our institution between 2014 and 2016. We measured the tumor occupancy ratio and noted the sagittal and axial location of the tumor in the preoperative MRI and performed the laminectomy and unilateral medial facetectomy. A primary outcome has been noted according to the gait disability score in the preoperative period and in the follow-up period of 1 year. In the statistical analysis, categorical variables were compared using a chi-square test, and an analysis of variance and student t tests were used for the continuous variables. P < .05 was considered statistically significant. RESULTS: The tumor occupancy ratio showed a significant association to the preoperative gait disability score (P < .001) and also predicted that the surgical outcome is much better in patients with tumors with a low tumor occupancy ratio than in patients with tumors with a high occupancy ratio (P < .001). No difference in the tumor occupancy ratio was noted in 2 different pathological tumors, and there was also no difference in the tumor occupancy ratio at different sagittal and axial locations of the tumor. CONCLUSION: Tumor occupancy ratio has shown a significant impact on the preoperative course and also predicts the surgical outcome in patients with IDEM tumors. Hence, it is an important imaging characteristic to prognosticate the outcome in IDEM tumors and should be noted in each case.

Rapidly growing diffuse neurofibroma of the scalp with calvarial defect in a young woman: A rare entity.

BACKGROUND: A diffuse neurofibroma, a variant of neurofibroma, most commonly occurs in young adults and involves the head and neck. In the absence of neurofibromatosis, associated calvarial defect with these swellings is rarely seen. CASE DESCRIPTION: An 18-year-old woman presented with a history of rapidly progressive painless large swelling over the bilateral parieto-occipital region of scalp. It was soft and boggy with brownish discoloration of overlying skin. Imaging study showed brilliantly enhancing diffuse lesion involving the bilateral parieto-occipital region of scalp and extending into the extradural region. She underwent excision of lesion. Postoperatively, she developed flap necrosis and it was allowed to heal with the secondary intention. The biopsy findings were consistent with neurofibroma. The patient is on regular follow-up, without any evidence of recurrence at 1 year. CONCLUSION: Rapidly growing solitary diffuse neurofibroma is rare in children and adolescents. Preoperative diagnosis may be difficult and surgical treatment needs to be individualized. These patients need regular follow-up for early detection of recurrence.

Hemorrhage in long segment cervical schwannoma; case report and literature review.

BACKGROUND: Although hemorrhages associated with cervical and thoracic intraspinal schwannomas are typically localized to the subarachnoid hemorrhages (SAH) or subdural hemorrhages (SDH) compartments, rare intratumoral bleeds may also occur. METHODS: In the literature, we found and analyzed multiple factors for 13 cases (e.g., epidemiological, clinical, and pathological) of cervical schwannomas with intratumoral hemorrhages (ITH). We added the 14th case of a 35-year-old female with along segment cervical schwannoma with ITH who presented with acute quadriplegia and respiratory decompensation. RESULTS: These 14 patients averaged 51.77 years of age, 60% were male, and the tumor involved 2.83 segments. The incidence of SAH and ITH was noted in five cases each, while SDH's were very rare. The pathological characteristics were consistent with the diagnosis of cellular schwannomas with S-100 positivity. The clinical outcomes were good (100%) in all the cases, including the one presented (modified McCormick score III). CONCLUSION: Cervical schwannomas with ITH are rare, and the surgical outcomes in such patients are good-excellent (>90%). The histopathology is always of prime importance and decisive in establishing and confirming the etiology of such ITH.